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常见变异型免疫缺陷性肠病及其不可预测的活检结果:并非一切都是非黑即白。

Common Variable Immunodeficiency Enteropathy and Its Unpredictable Biopsy Findings: Not Everything Is Black and White.

作者信息

Osama Md Ali, Dhawan Shashi, Rao Seema, Arora Anil Kumar

机构信息

Lady Hardinge Medical College, New Delhi, India.

Sir Gangaram Hospital, New Delhi, India.

出版信息

Middle East J Dig Dis. 2022 Oct;14(4):478-482. doi: 10.34172/mejdd.2022.310. Epub 2022 Oct 30.

DOI:10.34172/mejdd.2022.310
PMID:37547502
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10404095/
Abstract

Common variable immunodeficiency syndrome (CVID) is a diverse entity characterized by hypogammaglobinemia and a propensity for recurrent infections. Involvement of the gastrointestinal tract has a variable manifestation ranging from asymptomatic involvement to florid signs and symptoms. Due to these incongruous findings, multiple concurrent biopsies are to be done for tissue diagnosis. Here, we present two cases diagnosed with CVID on the basis of clinical findings, lab investigations, and morphological features on biopsy.

摘要

普通可变型免疫缺陷综合征(CVID)是一种具有多种表现的疾病,其特征为低丙种球蛋白血症和易反复感染。胃肠道受累的表现各异,从无症状到明显的体征和症状都有。由于这些不一致的发现,需要进行多次同步活检以进行组织诊断。在此,我们报告两例根据临床表现、实验室检查及活检的形态学特征诊断为CVID的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a95/10404095/9379771bff3a/mejdd-14-478-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a95/10404095/832287591369/mejdd-14-478-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a95/10404095/9379771bff3a/mejdd-14-478-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a95/10404095/832287591369/mejdd-14-478-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6a95/10404095/9379771bff3a/mejdd-14-478-g002.jpg

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J Can Assoc Gastroenterol. 2020 Aug;3(4):162-168. doi: 10.1093/jcag/gwz004. Epub 2019 Feb 25.
2
Shorter Diagnostic Delay in Polish Adult Patients With Common Variable Immunodeficiency and Symptom Onset After 1999.波兰成年普通可变免疫缺陷患者的诊断延迟时间更短,症状出现于 1999 年后。
Front Immunol. 2020 May 27;11:982. doi: 10.3389/fimmu.2020.00982. eCollection 2020.
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Spectrum of Hepatic Manifestations of Common Variable Immunodeficiency.
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Am J Surg Pathol. 2020 May;44(5):617-625. doi: 10.1097/PAS.0000000000001452.
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Liver Transplantation in Adults With Liver Disease Due to Common Variable Immunodeficiency Leads to Early Recurrent Disease and Poor Outcome.患有常见可变免疫缺陷所致肝病的成人进行肝移植会导致疾病早期复发且预后不良。
Liver Transpl. 2018 Nov;24(11):1622-1626. doi: 10.1002/lt.25343.
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Delayed Diagnosis and Complications of Predominantly Antibody Deficiencies in a Cohort of Australian Adults.澳大利亚成年人队列中以抗体缺陷为主的疾病的延迟诊断和并发症。
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